Download A case report of chronic sclerosing panniculitis

Journal of Pakistan Association of Dermatologists 2010; 20: 246-248.
Case Report
A case report of chronic sclerosing panniculitis
Hadiuzzaman*, M. Hasibur Rahman*, Nazma Parvin Ansari**, Aminul Islam†
*Department of Dermatology, Community Based Medical College, Bangladesh, Mymensingh,
Bangladesh.
**Department of Pathology, Community Based Medical College, Bangladesh, Mymensingh,
Bangladesh
†Department of Medicine, Community Based Medical College, Bangladesh, Mymensingh,
Bangladesh
Abstract Sclerosing panniculitis is a fibrotic process that usually occurs on the legs, commonly in women
older than 40. The principal features are indurated woody plaques with erythema, edema,
telangiectasia, and hyperpigmentation. Although the exact pathogenesis is uncertain, it is thought
to occur as a result of ischemic changes. We present a 28-year-old married female who had a 10year history of painful sclerotic plaques, repeated ulceration and healing with fibrosis of the both
lower legs and abdomen. Venogram and Doppler investigations were normal. Skin biopsy from the
edge of the ulcer demonstrated the feature of chronic sclerosing panniculitis. Satisfactory
improvement was found with methotrexate 7.5mg weekly for 4 months. No recurrence was noted
within 1 year follow up.
Key words
Sclerosing panniculitis, lipodermatosclerosis.
Case report
A 28-year-old married female presented to
dermatology outpatient, Community Based
Medical College, Bangladesh, with a 10-year
history of painful repeated ulceration and
healing with fibrosis of the both lower legs and
abdomen. This patient frequently visited
different doctors for his lesions; sometimes
healing was noted with conventional antibiotics
and other supportive medicine but no permanent
cure. The patient had been hospitalized
repeatedly for episodes of increased pain,
redness, oozing and swelling surrounding the
ulcers.
Address for correspondence
Dr. M. Hasibur Rahman,
Associate Professor of Dermatology,
Community Based Medical College,
Bangladesh, Mymensingh, Bangladesh.
E mai l: [email protected]
Mild swelling of the legs worse at the end of the
day was also reported. Tenderness of the ulcer
was worse with dependency. There was no
history of previous trauma to the area, joint
complaint, pancreatic disease, or other tender
nodular lesions or ulcerations. There was no
significant history of fever and night sweating.
All the members of family were fine. She took
different types of oral and injectable antibiotics
and even a therapeutic trial of antituberculous
therapy but improvement was not satisfactory.
Some
improvement
was
found
with
corticosteroid therapy but was followed by
recurrences.
On examination, there were multiple, mildly
tender, sclerotic/fibrotic plaques with scaling,
asymmetrically distributed over both lower legs
(Figure 1) and abdomen. Some oozing ulcerated
areas were noted over the plaques. There was no
regional lymphadenopathy. No associated
itching, hypoesthesia or loss of peripheral
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Journal of Pakistan Association of Dermatologists 2010; 20: 246-248.
Discussion
Sclerosing panniculitis also known as
‘lipodermatosclerosis’,
‘hypodermitis
sclerodermiformis’, and ‘stasis panniculitis’1 is a
skin and connective tissue disease. It is a form of
lower extremity panniculitis,2 an inflammation
of the subcutaneous fat.
Figure 1 A sclerotic plaque on left shin..
pulses was recorded.
On laboratory investigation, CBC, liver
functions, renal functions, and blood glucose
were within normal limit. Mantoux test showed
15mm induration after 72 hours. Skin biopsy
from the edge of the ulcer demonstrated a
thinned epidermis; the superficial dermis
showed sclerosis with angioplasia, patchy,
chronic inflammation, and pigment deposition.
Fibroadipose tissue with broad bands of
sclerosis
and
fibrosis
and
prominent
lipomembranous change with shrunken fat
lobules was present beneath the dermis. In
addition, other areas showed a lobular
panniculitis with foamy macrophages and
scattered lymphocytes and plasma cells. The
biopsy was consistent with sclerosing
panniculitis/lipodermatosclerosis.
The patient was given methotrexate 7.5 mg
weekly for 4 months with periodic monitoring of
liver function tests, renal function tests and
CBC. The patient subsequently had steady,
gradual healing over 4 months without
complications.
Pain may be the first noticed symptom.2 People
with lipodermatosclerosis have tapering of their
legs above the ankles, forming a constricting
band resembling an inverted coke bottle.2,4 In
addition,
there
may
be
brownish-red
4
pigmentation and induration.
The exact cause of lipodermatosclerosis is
unknown.2,5 Venous disease, such as venous
incompetence, venous hypertension, and body
mass (obesity) may be relevant to the underlying
pathogenesis.2
Increased blood pressure in the veins (venous
hypertension) can cause diffusion of substances,
including fibrin, out of capillaries. Fibrotic
tissue may predispose the tissue to ulceration.
Recurrent ulceration and fat necrosis is
associated with lipodermatosclerosis. In
advanced cases, the proximal leg swells from
chronic venous obstruction and the lower leg
shrinks from chronic ulceration and fat necrosis
resulting in the inverted coke bottle appearance
of the lower leg.6
Lipodermatosclerosis is most commonly
diagnosed in middle-aged women.2 It is usually
diagnosed clinically, and skin biopsy is not done
routinely. Dermatopathological changes mainly
affect the fat and depend on the stage of the
disease. Early lesions show an infiltrate of
lymphocytes and areas of tissue necrosis in the
subcutis septa; in intermediate lesions there is a
mixed infiltrate of white cells and new fibrous
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Journal of Pakistan Association of Dermatologists 2010; 20: 246-248.
tissue in the septa. Fibrous zones are present in
the fat. Late lesions show marked fibrosis in the
fat with diminished or absent inflammatory
cells. Changes in the dermis include a mixed
inflammatory cell infiltrate, increased fibrous
cells, atrophy, or both, and tortuous thick-walled
veins. Fibrin cuffs are seen around the
capillaries by direct immunofluorescence. The
histology stages correlate well with the two
stages seen clinically.
•
•
•
•
Fibrinolytic agents such as stanozolol,
fibrinolytic agents use an enzymatic
action to help dissolve blood clots.2,3,4,7
Pentoxyfylline to increase blood flow
Clobetasol
propionate
(ultrapotent
topical
steroid)
or
intralesional
triamcinolone injections to reduce
inflammation
Capsaicin to reduce pain
References
Blood tests are not usually required in
lipodermatosclerosis but coagulation may be
tested.
Ultrasound scans and magnetic resonance
imaging may be used to define the extent of the
disease and to determine whether there is a role
for vascular surgery.
The most important part of management is
compression therapy to correct venous stasis.
Management may also include:
•
•
•
•
•
Vein surgery, endovenous laser ablation
or sclerotherapy for varicose veins.
Leg elevation,
Elastic compression stockings, and bed
rest
Weight reduction
Ultrasound therapy
1.
2.
3.
4.
5.
6.
7.
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